At the recent Irish Haemophilia Society AGM, one of the guest speakers, Dr. Craig Kessler from Georgetown University Medical Centre inWashington, DC. Delivered a lecture on the topic of Ageing and Haemophilia. This is a topic which the Irish Haemophilia Society will be addressing in more detail in the coming years. Our new Strategic Plan allows for the production of a specific publication on Ageing and Haemophilia which is targeted to be produced in 2012. In 2013 we are planning to hold a special conference on Ageing and Haemophilia.
We are now seeing the first generation of people with haemophilia who are going to live long enough to start experiencing the normal problems that men in middle and older age develop with their health. Dr. Kessler pointed out that the major causes of death in American men are cardiovascular disease and cancer. In the haemophilia population, in the 1980s and 1990s, HIV and Aids were the major causes of death in people with haemophilia in developed countries ( untreated or inadequately treated bleeding continues to be the major cause of death in people with haemophilia in developing countries). In the last ten years we have seen a shift to a point where one of the leading causes of death is now liver disease, related to Hepatitis C. Hopefully, with new and better treatments emerging in the coming years for Hepatitis C, this trend may be reversed. For many men with haemophilia over the age of 45, the whole concept of issues relating to ageing and the impact of haemophilia now become important.
Risks relating to heart disease, cancer, kidney disease, diabetes, respiratory diseases and mental health problems need to be addressed in the haemophilia population, as with any other population. Several studies published in Europe have demonstrated a lower risk of cardiac disease in people with haemophilia than in males in the normal population. This was long thought to be the case due perhaps to the fact that as the blood of a person with haemophilia does not clot as readily, there will be a consequent lower risk of heart attack due to a blood clot. However, this potential advantage, if it was real, is minimised by the use of factor concentrates, whereby the person would have a more normal factor level for a greater proportion of the time.
Indeed, Dr. Kessler showed data from a study which was carried out in the United States in the mid 1990s showing that people with haemophilia have a higher relative risk of heart disease, liver disease, renal disease and non HIV or liver related cancers. The data from the USA is at odds with the data published from European studies and this difference can not be readily explained. It may be due to the different methods of data collection or it may be due a different lifestyle pattern in the United States. In data published in the United Kingdom in 2007 the average life expectancy of all males in the UK in 1999 was 78 wheras the average life expectancy of males with severe haemophilia was 63 and of those with mild or moderate haemophilia was 75. Data from the UK also showed that people with haemophilia have a higher risk than the general population for death from bleeding or from intracranial haemorrhage (that is not unexpected). They also had a higher risk than average of death from liver cancer, from liver disease or from Hodgkin’s disease. The increased deaths from liver cancer or liver disease are not unexpected given the prevalence of Hepatitis C in the population. The increased risk of Hodgkin’s disease was more surprising and Dr. Kessler postulated that this may be associated with the fact that prior to the 1990s people with haemophilia tended to be treated with large quantities of intermediate purity plasma derived factor concentrates, which contained a lot of diverse proteins. These could have increased the challenges to the immune system and could be linked to the later increased risk of diseases such as Hodgkin’s. Interestingly, the United Kingdom data from 2007 showed a lower risk of heart disease in the haemophilia population when compared to the general male population.
Dr. Kessler also discussed the potential impact of Highly Active Anti Retro Viral Therapy (HAART) in HIV Positive people with haemophilia and the fact that wheras this therapy has been excellent at controlling HIV it may lead to an increased risk of dementia. Many of the therapies used to control and manage risks of heart disease such as the use of statins or aspirin have to be looked at in the light of the underlying haemophilia. The importance of good diet and an appropriate exercise regime were also discussed. There is an emerging issue in the general population, known as Metabolic X Syndrome, which includes obesity, high blood pressure, high fasting blood glucose, high levels of cholesterol and triglycerides (fats) in the blood and a fatty liver. This combination is a recipe for disaster and certainly will put any person with this combination (including persons with haemophilia) at higher risk of heart disease, diabetes or serious liver disease.
The topic of Ageing and Haemophilia may seem rather depressing and the news may seem to be uniformly bad. That is not the case. Ageing is inevitable. What we are seeing is, for the first time, the beginning of the accumulation of evidence and data on the impact of Ageing on a population of men with haemophilia. Heart disease, cancers, liver disease, kidney disease, diabetes are all conditions which increase in a population as they get older. It would be nice to think that people with haemophilia will never age and will live forever , sadly that is not the case. People with haemophilia need to be proactive in relation to their health. We should all ensure that we are tested on a regular basis for any abnormalities in our cholesterol, blood pressure, glucose levels and that screening for prevalent male cancers such as colorectal and prostate cancer are carried out at appropriate intervals and not ignored. We should look to maintain a balanced lifestyle to include diet and appropriate exercise and we should work with the healthcare professionals to maintain good health. I know that the National Centre for Hereditary Coagulation Disorders (NCHCD) will be examining the whole issue of Ageing and Haemophilia and looking at an appropriate clinical response in relation to helping people with haemophilia to prevent the onset of diseases of ageing, to diagnose and problems early, to monitor and maintain health and to treat appropriately. This is a topic we will be returning to on future occasions.